The myositis syndromes include polymyositis, dermatomyositis (DM), necrotizing myopathy, inclusion body myositis (IBM), antisynthetase syndrome and overlap syndromes with myositis. These syndromes mostly occur in middle-aged patients, while juvenile DM occurs in children and adolescents. Patients mostly show a subacute weakness and myalgia in the upper and lower limbs, the diagnosis is based upon these clinical findings in combination with muscle biopsy results and specific serum autoantibodies. In recent years, research achieved a better understanding about the molecular mechanism underlying the myositis syndromes, as well as disease progress and extramuscular organ manifestations, such as interstitial lung disease and association with neoplasias. Treatment mainly consists of glucocorticosteroids and immunosuppressants. IBM is usually refractory to treatments. This review provides an overview of the current standards of treatment and new treatment options like monoclonal antibodies and new molecular therapies and their first results from clinical trials.

Purpose of Review Dysphagia is a common symptom in inflammatory myopathies. This review provides an overview on the epidemiology, clinical impact, and management of dysphagia in myositis. Relevant diagnostic tools and treatment strategies are discussed. Recent Findings Dysphagia can occur in any inflammatory myopathy, particularly in inclusion body myositis (IBM). It can lead to malnutrition or aspiration with subsequent pneumonia or even death. Dysphagia can be explored and monitored by patientreported outcome scales for swallowing. New diagnostic tools such as real-time MRI and oro-pharyngo-esophageal scintigraphy have been studied for assessing dysphagia. Botulinum toxin injection can alleviate dysphagia in IBM. High-dose glucocorticosteroids are considered a first-line treatment for dysphagia in all other myositis subforms. Summary Evaluation of dysphagia in myositis requires thorough clinical workup and appropriate instrumental procedures. Treatment options are available for dysphagia, but controlled trials and consensus on best patient care are required for this important symptom.

Inflammatory myopathies, including polymyositis (PM), dermatomyositis (DM), inclusion body myositis (IBM), necrotizing myopathy (NM), antisynthetase syndrome (ASS) and overlap myositis (OM), in short myositis, are rare diseases. All forms of myositis have progressive muscle weakness in common, with each subtype characterized by different autoantibody profiles, histological findings and extramuscular manifestations. Due to better understanding of the pathogenesis of the muscle inflammation in myositis, new molecular pathways for targeted therapy have been discovered. Current therapies aim at different components of the innate or the adaptive immune response. Additionally, non-inflammatory mechanisms in myositis have come into focus as possible treatment targets. The use of therapeutical antibodies in myositis has been examined in various clinical studies, several of them randomized controlled ones: Depletion of B-cells by rituximab has been established as treatment of refractory myositis. IVIG, an antibody therapy in the wider sense, has now been licensed for DM following a recent positive clinical trial. Negative study results were reported in randomized trials with infliximab, sifalimumab and bimagrumab. Studies on basiliximab and eculizumab are currently underway, and are expected to yield results in a couple of years. Despite some promising results of clinical studies with antibody therapy in myositis, further research is crucial to optimize the treatment for this debilitating disease and to find treatment alternatives for treatment-refractory patients.

: Inflammatory myopathies, in short, myositis, are heterogeneous disorders that are characterized by inflammation of skeletal muscle and weakness of arms and legs. Research over the past few years has led to a new understanding regarding the pathogenesis of myositis. The new insights include different pathways of the innate and adaptive immune response during the pathogenesis of myositis. The importance of non-inflammatory mechanisms such as cell stress and impaired autophagy has been recently described. New target-specific drugs for myositis have been developed and are currently being tested in clinical trials. In this review, we discuss the mechanisms of action of pharmacological standards in myositis and provide an outlook of future treatment approaches.

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Zusammenfassung Hintergrund Die Diagnose und Behandlung von Patienten mit immunvermittelten Polyneuropathien ist aufgrund der Heterogenität der Erkrankungen herausfordernd. Ziel der Arbeit Ein aktueller epidemiologischer Überblick über die Versorgungssituation von Patienten mit immunvermittelten Polyneuropathien innerhalb des deutschen Neuritis-Netzwerks „Neuritis Netz“. Material und Methoden Es erfolgte eine Umfrage in neun deutschen neurologischen Zentren, die auf die Betreuung von Patienten mit Immunneuropathie spezialisiert sind. Wir erfassten Diagnose, Vorgehen in der Diagnostik und Nachsorge, typische Symptome bei Manifestation und im Krankheitsverlauf sowie Therapiedaten. Ergebnisse Die Erhebung umfasst Daten von 1529 jährlich behandelten Patienten mit Immunneuropathien, 1320 davon mit chronisch inflammatorisch demyelinisierender Polyneuropathie (CIDP). Die Diagnostik umfasste fast immer Lumbalpunktionen sowie Elektroneuro- und -myografien entsprechend den aktuellen Leitlinien. Der Einsatz von Ultraschall, Biopsie und MRT war unterschiedlich. Wichtigster klinischer Parameter zum Therapiemonitoring in allen Zentren war die motorische Funktion in den klinischen Nachuntersuchungen. Zur Erhaltungstherapie wurde bei rund 15 % der Patienten ein breites Spektrum unterschiedlicher Immunsuppressiva eingesetzt. Diskussion Die Studie liefert wichtige epidemiologische Daten zur aktuellen Versorgungsituation von Patienten mit Immunneuropathien in Deutschland. Die Weiterentwicklung spezifischer Empfehlungen zur Therapie und Nachverfolgung von CIDP-Patienten ist notwendig, um einen einheitlichen Standard der Patientenversorgung zu gewährleisten. Dieses wird durch die strukturierte Zusammenarbeit von Exzellenzzentren wie dem deutschen Neuritis Netz erheblich unterstützt.